Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children

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dc.contributor.authorTrautmann, Agnes
dc.contributor.authorSchnaidt, Sven
dc.contributor.authorLipska-Zietkiewicz, Beata S.
dc.contributor.authorBodria, Monica
dc.contributor.authorOzaltin, Fatih
dc.contributor.authorEmma, Francesco
dc.contributor.authorAnarat, Ali
dc.contributor.authorMelk, Anette
dc.contributor.authorAzocar, Marta
dc.contributor.authorOh, Jun
dc.contributor.authorSaeed, Bassam
dc.contributor.authorGheisari, Alaleh
dc.contributor.authorCaliskan, Salim
dc.contributor.authorGellermann, Jutta
dc.contributor.authorHiguita, Lina Maria Serna
dc.contributor.authorJankauskiene, Augustina
dc.contributor.authorDrozdz, Dorota
dc.contributor.authorMir, Sevgi
dc.contributor.authorBalat, Ayse
dc.contributor.authorSzczepanska, Maria
dc.contributor.authorParipovic, Dusan
dc.contributor.authorZurowska, Alexandra
dc.contributor.authorBogdanovic, Radovan
dc.contributor.authorYilmaz, Alev
dc.contributor.authorRanchin, Bruno
dc.contributor.authorBaskin, Esra
dc.contributor.authorErdogan, Ozlem
dc.contributor.authorRemuzzi, Giuseppe
dc.contributor.authorFirszt-Adamczyk, Agnieszka
dc.contributor.authorKuzma-Mroczkowska, Elzbieta
dc.contributor.authorLitwin, Mieczyslaw
dc.contributor.authorMurer, Luisa
dc.contributor.authorTkaczyk, Marcin
dc.contributor.authorJardim, Helena
dc.contributor.authorWasilewska, Anna
dc.contributor.authorPrintza, Nikoleta
dc.contributor.authorFidan, Kibriya
dc.contributor.authorSimkova, Eva
dc.contributor.authorBorzecka, Halina
dc.contributor.authorStaude, Hagen
dc.contributor.authorHees, Katharina
dc.contributor.authorSchaefer, Franz
dc.date.accessioned2019-10-27T11:05:18Z
dc.date.available2019-10-27T11:05:18Z
dc.date.issued2017
dc.departmentEge Üniversitesien_US
dc.description.abstractWe investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models. Complete and partial remission of proteinuria within 12 months of disease onset occurred in 24.5% and 16.5% of children, respectively, with the highest remission rates achieved with calcineurin inhibitor based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52% in children with histopathologic findings of minimal change glomerulopathy and FSGS, respectively. Five-year ESRD-free survival rate was 21% for diffuse mesangial sclerosis. IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. Our findings suggest that responsiveness to initial IIS and detection of a hereditary podocytopathy are prognostic indicators of favorable and poor long-term outcome, respectively, in children with steroid-resistant nephrotic syndrome. Children with multidrug-resistant sporadic disease show better renal survival than those with genetic disease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established.en_US
dc.description.sponsorshipE-Rare (German Ministry of Education and Research); Polish Ministry of Science and Education grant [N402631840]; German Research FoundationGerman Research Foundation (DFG) [Scha 477/11-1]; Scientific and Technological Research Council of TurkeyTurkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK) [108S417]; European Union (EU) Seventh Framework Programme (EURenOmics) grant [2012-305608]en_US
dc.description.sponsorshipThe PodoNet project has been made possible by support received from E-Rare (German Ministry of Education and Research), European Union (EU) Seventh Framework Programme (EURenOmics) grant 2012-305608, Polish Ministry of Science and Education grant N402631840, German Research Foundation grant Scha 477/11-1, and Scientific and Technological Research Council of Turkey grant 108S417.en_US
dc.identifier.doi10.1681/ASN.2016101121en_US
dc.identifier.endpage3065en_US
dc.identifier.issn1046-6673
dc.identifier.issn1533-3450
dc.identifier.issue10en_US
dc.identifier.pmid28566477en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage3055en_US
dc.identifier.urihttps://doi.org/10.1681/ASN.2016101121
dc.identifier.urihttps://hdl.handle.net/11454/31651
dc.identifier.volume28en_US
dc.identifier.wosWOS:000412042200025en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherAmer Soc Nephrologyen_US
dc.relation.ispartofJournal of the American Society of Nephrologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleLong-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Childrenen_US
dc.typeArticleen_US

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